Sickle Cell

About Sickle Cell Anemia

The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.

Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.

“Inherited” means that the disease is passed by genes from parents to their children. SCD is not contagious. A person cannot catch it, like a cold or infection, from someone else.

People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.

Hemoglobin SC disease and hemoglobin Sβ thalassemia (thal-uh-SEE-me-uh) are two other common forms of SCD.

Overview

Cells in tissues need a steady supply of oxygen to work well. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it to all the tissues of the body.

Red blood cells that contain normal hemoglobin are disc shaped (like a doughnut without a hole). This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen.

Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape.

Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can’t reach nearby tissues.

Sickle cell disease is a life-long illness. The severity of the disease varies widely from person to person.

In high-income countries like the United States, the life expectancy of a person with SCD is now about 40–60 years. In 1973, the average lifespan of a person with SCD in the United States was only 14 years. Advances in the diagnosis and care of SCD have made this improvement possible.

At the present time, hematopoietic stem cell transplantation (HSCT) is the only cure for SCD. Unfortunately, most people with SCD are either too old for a transplant or don’t have a relative who is a good enough genetic match for them to act as a donor. A well-matched donor is needed to have the best chance for a successful transplant.

There are effective treatments that can reduce symptoms and prolong life. Early diagnosis and regular medical care to prevent complications also contribute to improved well-being.

 

What is AA AS and SS genotype?
What is a Geno type? Your genotype is your complete heritable genetic identity; the sum total of genes transmitted from parent to offspring. There are four hemoglobin genotypes (hemoglobin pairs/formations) in humans: AA, AS, SS and AC (uncommon). SS and AC are the abnormal genotypes or the sickle cells.

What is AA in sickle cell?
Genetics. Normally, a person inherits two copies of the gene that produces beta-globin, a protein needed to produce normal hemoglobin (hemoglobin A, genotype AA). A person with sickle cell trait inherits one normal allele and one abnormal allele encoding hemoglobin S (hemoglobin genotype AS).

Compatible genotypes for marriage are:
AA marries an AS. You’ll end up with kids with AA and AS which is good. But sometimes if you’re not lucky all the kids will be AS which limits their choice of partner. AS and AS should not marry, there is every chance of having a child with SS.

Who gets sickle cell?
Sickle cell disease is more common in certain ethnic groups, including: People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene) Hispanic-Americans from Central and South America. People of Middle Eastern, Asian, Indian, and Mediterranean descent.

Can a child have sickle cell trait if neither parent has it?
With a few exceptions, a child can inherit sickle cell disease only if both parents have one gene for sickle cell hemoglobin. The most common situation in which this occurs is when each parent has one sickle cell gene. In other words, each parent has sickle cell trait.

What are the signs of sickle cell in babies?
This condition happens when the sickle cells block blood flow in your child’s hands and feet. Signs and symptoms include fever and pain, swelling or coldness in the hands and feet.

Signs and symptoms may include:
Fever.
Coughing.
Breathing problems.
Pain in the bones.
Headaches.

Can a person with sickle cell have a baby?
Can Women With Sickle Cell Disease Have A Healthy Pregnancy? Yes, with early prenatal care and careful monitoring throughout the pregnancy, a woman with SCD can have a healthy pregnancy. However, women with SCD are more likely to have problems during pregnancy that can affect their health and that of their unborn baby.

Is Sickle Cell curable?
Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they’re not done very often because of the significant risks involved. Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells.

What food is good for sickle cell?
Eat from a rainbow of fruits and vegetables paired with grains, proteins (such as eggs, fish, chicken, lean meat, beans or tofu) and nuts. Get plenty of calcium-rich foods such as low-fat or fat-free milk, yogurt, and cheese, leafy green vegetables and calcium-fortified foods such as soymilk, orange juice and tofu.

Is Sickle Cell tested at birth?
SCT), as early as 24-48 hours after birth. are screened for sickle cell status as part of the newborn screening program. a condition reported but you need more testing by your baby’s doctor to know for sure. Both SCT and SCD are conditions that are genetically inherited or passed down from your parents